Cognitive and Functional Improvement in a Pediatric Patient with Acute Disseminated Encephalomyelitis Following Methylphenidate Treatment: A Case Report

AbstractSetting: Acute rehabilitation hospital. Patient: A 4-year-old girl with acute disseminated encephalomyelitis (ADEM). Case Description: The patient was admitted to the brain injury unit of an acute rehabilitation hospital 10 days after diagnosis and treatment at a pediatric hospital. Residual effects from the disease process in this patient included flaccid limbs and generalized weakness with total dependence required for many activities of daily living (ADLs), cognitive slowing with decreased verbalization and play, and lethargy. After 1 week of inpatient rehabilitation, patient made no functional or cognitive gains. Plans were made to start patient on a neurostimulant. Patient was on daily dosing of amantadine for 1 day; however, this was not tolerated secondary to severe nausea and vomiting. Daily dosing of 2.5 mg oral methylphenidate (MPH) was initiated on rehab day 10. This dosage was titrated to 2.5 mg twice daily dosing (administered at 7 AM and noon) on rehab day 14. Assessment/Results: Prior to the administration of MPH, the patient remained lethargic and made poor functional gains during her acute inpatient rehabilitation stay. Within 24 hours of the initiation of MPH, the patient’s condition markedly improved, including increased alertness and ability to communicate, increased age-appropriate play, and improved performance of ADLs. Discussion: MPH is a mild central nervous system stimulant that is indicated by the FDA for use in the treatment of attention deficit disorder and narcolepsy. MPH has also been used off label for decades for the treatment of cognitive dysfunction in traumatic brain injury in both the adult and pediatric population. MPH aided in improving arousal and attention in this patient, which contributed to the efficacy of her therapy sessions. Conclusion: MPH was used successfully as a neurostimulant in this pediatric patient with ADEM. Further investigation and controlled studies would be warranted in the future.

Key Words: Acute disseminated encephalomyelitis; Methylphenidate; Pediatrics; Rehabilitation

Figure 1: Timeline of patient’s course

Jessica L. Colyer, MD and Chad A. Walters, DO University of Kentucky Department of Physical Medicine and Rehabilitation, Lexington, KY

Figure 2: Representative MRI images of patient’s brain (FLAIR and T2 weighed)

DiscussionAcute disseminated encephalomyelitis (ADEM) is an immune-mediated CNS demyelinating disease that usually follows a benign infection in healthy young people and often presents as an acute encephalopathy with multifocal neurologic signs and deficits

Most patients are treated via supportive measures and often with IV methylprednisolone for 3-5 days and/or administration of IVIg—sequelae vary, but most patients eventually recover from this disease process.

Much study has been performed on neurostimulant use in the TBI population regarding general cognitive function, attention, processing time and executive functioning; however, study of the use of neurostimulants following infection and auto-immune disorders has been very limited.

Methylphenidate was used successfully as a neurostimulant in this pediatric patient with ADEM.

Methylphenidate aided in improving arousal and attention in this patient, which contributed to the efficacy of her therapy sessions.

Further investigation and controlled studies would be warranted in the future

References1.Young NP, Weinshenker BG, and Lucchinetti CF. Acute

disseminated encephalomyelitis: current understanding and controversies. Semin Neurol 2008; 28:84-94.

2.Leake JA, Albani S, Kao AS, et al. Acute disseminated encephalomyelitis in childhood: epidemiologic, clinical and laboratory features. Pediatr Infect Dis J 2004; 23(8):756-764

3.Weber P, Lütschg J. Methylphenidate treatment. Pediatr Neurol 2002 Apr;26(4):261-6

4.Hornyak JE, Nelson VS, and Hervitz EA. The use of methylphenidate in paediatric traumatic brain injury. Pediatr Rehabil 1997 Jan-Mar;1(1):15-7

5.Warden DL, Gordon B, McAllister TW, et al. Guidelines for the Pharmacologic Treatment of Neurobehavioral Sequelae of Traumatic Brain Injury. J Neurotrauma 2006; 23(10): 1468-1501

Table 1: Descriptives of patient function on admission to acute rehab •Poor sitting and standing balance (static/dynamic)

•Total assist (<25% patient effort) for standing

•Max to total assist for initiating one step in gait

•Max to total assist for lift transfers

•Total assist for grooming, bathing, dressing, eating

•Non-verbal—cries only, will nod yes/no

•Makes eye contact—has normal visual tracking

•Decreased p.o. intake, only nutrition via NG tube

•Impaired gross motor function—no functional 3 point, pincer or tip pinch grasp

•Fluctuating attention and arousal

•Increased cognitive processing time

•Impaired memory/sequencing, problem solving

Table 2: Descriptives of patient function on discharge from rehab •Sits and stands independently

•Walks 150’ level surface with modified independence using handheld assist

•Contact guard to stand by assist in transfers

•Grooming and bathing with family assist

•Dressing at chair level with set-up

•Age-appropriate socialization, play, and communication

•Able to assist with clean up after play

•Fair to good gross motor function

•Fair pincer grasp—continued weakness

•Alert—decreased attention with distraction

•Fair to good memory, can sequence ABCs and name colors accurately

One month prior to hospitalization• Diagnosed with sinusitis by PCP• Started on amoxicillin• Developed nausea and vomiting• Admitted to local hospital for rehydration

Admitted to Children’s Hospital• Due to continued fatigue, poor p.o. intake, PCP transferred to higher acuity facility

• On admission, patient had fever, headache, abdominal pain, irritability, and generalized weakness

Acute Care Hospital Day 2• Basic labs and CSF studies are normal

• CT of head is normal• Serology negative for Lyme disease, West Nile disease, Enterovirus, EBV IgG/IgM, Ehrlichia, Tularemia, St Louis Encephalitis, Eastern Equine Encephalitis, Western Equine Encephalitis, California Encephalitis, Bartonella

Acute Care Hospital Day 3• EEG shows excessive diffuse cerebral dysfunction for age.

• MRI indicative of ADEM• Patient treated with supportive care—Dobhoff for feeding, Foley for bladder management

Admission to Acute Rehab (HD12)• Patient irritable and fussy—uncooperative with exam and did not follow commands

• Tetraparesis with normal DTRs• Unable to assess muscle strength 2° to cognitive limitations

• Light touch grossly intact, but hypersensitive to all stimuli

Rehab Day 10• By rehab day 7, no cognitive or functional gains have been made

• Methylphenidate 2.5 mg qAM initiated (titrated to qAM and qnoon by day 14)

Rehab Day 11• Patient made attempts at communication through speaking (stating “no”), was able to roll with mod assist, and supported herself prone on elbows for 5 minutes with mod assist

Rehab Day 15• All therapies noticed an increase in patient’s level of arousal with increased ability to follow commands

• Patient had much improved trunk control, tolerated standing with max-mod assist for 20 minutes, lay prone on elbows for 15 minutes, was able to do 10 push-ups, walked 15’ with max assist.

Discharge from Acute Rehab• Patient continued to make good cognitive and functional progress and was discharged from inpatient rehab on day 38.

There are ill-defined areas of high signal seen in bilateral basal ganglia without evidence of contrast enhancement. These areas of abnormality include bilateral putamen, caudate nucleus, lentiform nucleus and also additionally the subcortical white matter in the frontal region and the frontal lobe appears involved.